Published: 2022-11-25

An unusual presentation of catastrophic antiphospholipid antibody syndrome in the background of sepsis

Sai C. Hakeem, Akash T. Oommen, Sandeep Surendran, Sumanth Madan


A 59-year-old female presented with complaints of gangrenous changes over right ring finger and reduced renal output. She was in hypotension and had to be started on ionotropes. She also had acute kidney injury and was initiated on hemodialyis. Inspite of culture directed antibiotics and amputation of the necrotic region, her condition worsened. Considering the acute multisystem worsening, i.e., less than a week, concomitant autoimmune etiology was considered. Antiphospholipid antibodies were positive. Her tissue biopsy was suggestive of vasculitis. Hence the diagnosis of catastrophic antiphospholipid antibody syndrome (CAPS) was made. Quick recognition and appropriate treatment play a cornerstone in treatment of CAPS. She was pulsed with methylprednisolone and also treated with intravenous immunoglobulins and anticoagulants. She showed remarkable improvement and responded to the treatment. CAPS should always be kept as a differential in case of multisystem acute deterioration even in the background of sepsis. The treatment is a big challenge to physicians given the associated mortality rate if not briskly treated.


Catastrophic antiphospholipid antibody syndrome, Sepsis, Gangrene

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Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, Khamashta MA, Shoenfeld Y; Catastrophic Antiphospholipid Syndrome Registry Project Group. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530-4.

Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A, et al. Catastrophic antibody syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998;77:195-207.

Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014;3(1):9-17.

Rodríguez-Pintó I, Espinosa G, Erkan D, Shoenfeld Y, Cervera R; CAPS Registry Project Group. The effect of triple therapy on the mortality of catastrophic anti-phospholipid syndrome patients. Rheumatology (Oxford). 2018;57(7):1264-70.

Hai A, Aslam M, Ashraf TH. Symmetrical peripheral gangrene: a rare presentation of antiphospholipid syndrome. Intern Emerg Med. 2012;7:71-3.